Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common. Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization. Following the WHO 2000 classification, PNETs have been considered embryonal tumors composed of undifferentiated neuroepithelial cells with a capacity of differentiation into different cellular lines, such as astrocytic, ependymal, melanotic and muscular. They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system. The optimal treatment for these malignant tumors is not yet clear, although, over the years, radiotherapy has been considered the best treatment for spinal PNETs. The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy. The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.
The role in tumor spread and the prognostic value of metastasis in a prelaryngeal node were investigated in 124 cases of laryngeal and hypopharyngeal carcinoma selected from about 900 total laryngectomies and pharyngolaryngectomies. Metastasis in a prelaryngeal node was noted in 26 cases: 22 without capsular rupture (N+) and 4 with capsular rupture (N+R+). Vascular embolism (E+) was never noted. Patients with a metastatic prelaryngeal node had a tumor of the piriform sinus in 13 cases (50%), a transglottic tumor in 6, glotto-subglottic in 3, glottic in 2 and supraglottic in 2. They also more frequently had involvement of the cervical nodes (81%) and of the thyroid gland. The 5-year survival in these patients is very poor: only 3 patients (11.5%) were alive and free of disease. These data demonstrate the role of prelaryngeal nodes in tumors that affect the hypopharynx and the antero-inferior portion of the larynx and that directly reach this node through the anterior lymphatic peduncle.
Solitary fibrous tumors (SFT) are rare neoplasms of mesenchymal origin involving soft tissues, mainly serosal sites; the spinal cord location is uncommon. We report a case of SFT occurring in the thoracic spinal cord, discussing histological, ultrastructural and molecular aspects. A 75-year-old woman with an MRI suggesting a dorsal intracanalar lesion was admitted to our institution. T5-T7 laminectomies were performed and an intramedullary tumor was discovered. The tumor arose within the spinal cord and was completely removed. Tumor samples were processed for histological, ultrastructural and molecular analysis (comparative genomic hybridization [CGH], methylation status of O6-methylguanine-DNA methyltransferase [MGMT], p16, deleted in colorectal cancer [DCC] and death-associated protein kinase 1 [DAPK1]). The histological examination demonstrated a proliferation of spindle-shaped cells with a collagen-matrix background. Immunohistochemical staining was positive for vimentin and CD34 and negative for S-100 and epithelial membrane antigen. A histological diagnosis of SFT was made. The ultrastructural examination showed undifferentiated cells within a collagenous matrix and sparse extravascular basement membrane. CGH analysis revealed deletion of 9p21 and losses on 2q, 3p, 16q and 19q and gains on 7q; furthermore, no aberrant methylation pattern was found in the promoter region of MGMT, p16, DCC and DAPK1 genes. On the second-year follow-up, the patient was neurologically intact. The occurrence of SFT within the spinal cord parenchyma and its histological characteristics demonstrate that SFTs are not restricted to serosal surfaces. The course of spinal cord SFT is unknown and long-term follow-up is necessary. The histological, ultrastructural and molecular findings are important for the diagnosis and the authors provide a literature review of these aspects.
Central neurocytomas are low grade tumours usually located in the lateral ventricles next to Monro foramina. This paper reviews the literature on central neurocytomas observed in the last few years and discusses their clinical, histopathological, immunohistochemical and genetic characteristics. Important correlations between therapeutic strategies and biological findings as well as new genetic discoveries are also discussed. Two illustrative cases in which the authors report preliminary results about molecular analysis of some genetic markers are described.
The SARS-CoV-2 pandemic has dramatically changed our lives and habits. In just a few months, the most advanced and efficient health systems in the world have been overwhelmed by an infectious disease that has caused 3.26 million deaths and more than 156 million cases worldwide. Although the lung is the most frequently affected organ, the skin has also resulted in being a target body district, so much so as to suggest it may be a real “sentinel” of COVID-19 disease. Here we present 17 cases of skin manifestations studied and analyzed in recent months in our Department; immunohistochemical investigations were carried out on samples for the S1 spike-protein of SARS-CoV-2, as well as electron microscopy investigations showing evidence of virions within the constituent cells of the eccrine sweat glands and the endothelium of small blood vessels. Finally, we conduct a brief review of the COVID-related skin manifestations, confirmed by immunohistochemistry and/or electron microscopy, described in the literature.
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